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Differential impairment of catecholaminergic cell maturation and survival by genetic mitochondrial complex II dysfunction

Opened Access Differential impairment of catecholaminergic cell maturation and survival by genetic mitochondrial complex II dysfunction

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Autor: Díaz Castro, Blanca
Pintado, Carmelo Oscar
García Flores, Gloria Paula
López Barneo, José
Piruat Palomo, José Ignacio
Departamento: Universidad de Sevilla. Departamento de Fisiología Médica y Biofísica
Fecha: 2012
Publicado en: Molecular and Cellular Biology (Vol.32, Nº16, p.3347–3357)Molecular and Cellular Biology, 32 (16), 3347-3357.
Tipo de documento: Artículo
Resumen: The SDHD gene (subunit D of succinate dehydrogenase) has been shown to be involved in the generation of paragangliomas and pheochromocytomas. Loss of heterozygosity of the normal allele is necessary for tumor transformation of the affected cells. As complete SdhD deletion is lethal, we have generated mouse models carrying a “floxed” SdhD allele and either an inducible (SDHD-ESR strain) or a catecholaminergic tissue-specific (TH-SDHD strain) CRE recombinase. Ablation of both SdhD alleles in adult SDHD-ESR mice did not result in generation of paragangliomas or pheochromocytomas. In contrast, carotid bodies from these animals showed smaller volume than controls. In accord with these observations, the TH-SDHD mice had decreased cell numbers in the adrenal medulla, carotid body, and superior cervical ganglion. They also manifested inhibited postnatal maturation of mesencephalic dopaminergic neurons and progressive cell loss during the first year of life. These alterations were particularly...
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Tamaño: 4.534Mb
Formato: PDF

URI: http://hdl.handle.net/11441/17474

DOI: 10.1128/MCB.00128-12

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